8/16/2023 0 Comments Narcolepsy without cataplexySpontaneous evolution of the disease has not been much studied, but the phenotype and evolution seems variable : stability, disappearance of the symptoms, development of cataplexy (conversion to narcolepsy type 1), or change to idiopathic hypersomnia. This can be triggered by strong emotion, especially laughter. The disease has a negative impact on scholarly and professional performances. Sometimes narcolepsy also causes a sudden loss of muscle tone, known as cataplexy (KAT-uh-plek-see). Second-line treatments include methylphenidate, sodium oxybate, solriamfetol or amphetamines. Modafinil or pitolisant are the first line treatments with the best benefit/risk ratio. Treatment is only symptomatic, based on stimulant drugs and a good sleep hygiene (sufficient sleep duration at night is required, with scheduled short naps during the day). Rare familial cases have been reported however, the mode of inheritance is unclear. Other causes of sleepiness, including narcolepsy type 1, chronic insufficient sleep and idiopathic hypersomnia (especially the form without long sleep time), must be systematically taken into account. According to the ICSD 2nd Edition (ICSD-2), narcolepsy with cataplexy (NwithC), narcolepsy without cataplexy (Nw/oC), idiopathic hypersomnia with long sleep. Cataplexy usually develops approximately several weeks to months after the development of excessive daytime sleepiness. A pure clinical diagnosis is not possible due to the absence of pathognomonic symptom such as cataplexy, and daytime sleepiness being a non-specific symptom. Some people with narcolepsy do not have cataplexy, and cataplexy is not necessary for a diagnosis of narcolepsy. Nocturnal and daytime polysomnography demonstrates an average sleep latency of under eight minutes with at least two sleep onset rapid eye movement periods (SOREMP) on multiple sleep latency tests. Diagnostic methodsÄefinitive diagnosis requires the presence of clinical symptoms and characteristics polysomnography findings. The presence of the HLA DQB1*0602 allele is reported in 40% of cases (more than in the general population but less than in cases of narcolepsy type 1, 98%). Reduction of hypocretin-1 levels is found in the cerebrospinal fluid in 10-20% of cases, and in which case the disorder is reclassified narcolepsy type 1. The etiology is still unknown, probably because this disorder is heterogeneous. More research is needed to determine the exact triggers behind narcolepsy without cataplexy. It can also be a lifelong disease, with stable symptomatology. Narcolepsy with cataplexy is an auto-immune disorder. Narcolepsy type 2 has a variable phenotype and evolution, with sometimes improvement or even disappearance of the symptoms, rarely the development of cataplexy (conversion to narcolepsy type 1), or a change in the phenotype to idiopathic hypersomnia. Narcolepsy type 2 manifests generally between the age of 10 and 30 years old, although onset in childhood is rare. Narcolepsy type 2 prevalence numbers are controversial, sometimes higher and sometimes lower than those of narcolepsy type 1, depending on reports. No clear epidemiological data are available.
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